Long-term results of children diagnosed with idiopathic nephrotic syndrome; single center experience

Abstract

Aim: The aim of this study was to determine the long-term results of children followed up with a diagnosis of nephrotic syndrome in a single center. Material and Methods:The medical data of 33 patients aged between 6 months and 10 years who were diagnosed with idiopathic nephrotic syndrome in our center between January 2000 and December 2012 and followed up for a period of 2-12 years were reviewed (Gulhane Military Medical Academy Ethics committee, 07.11.2012/10). Results: The mean age of disease onset was 3.2±2.04 years (range: 0.5-10 years) and the mean follow-up period was 6±3.4 years (range: 2-12 years). Thirteen (39.4%) of the study group (or the patients) were female and 20 (60.6%) were male. Twenty seven (1.8%) of the patients were sensitive to steroid and 6 (18.1%) were resistant to steroid. Four (12.1%) of the steroid-resistant patients had steroid-dependent nephrotic syndrome, 5 (15.2%) had frequently relapsing nephrotic syndrome and 18 (54.5%) had rarely relapsing nephrotic syndrome. Histopathological diagnoses of six patients who underwent biopsy because of resistance to steroid were as follows: focal segmental glomerulosclerosis (n=3), C1q nephropathy (n=1), diffuse mesangial proliferation (n=1) and membraneous nephropathy (n=1). Fifteen (45.5%) patients entered into full remission and 2 (6%) patients developed chronic renal failure. Treatment complications including decreased bone mineral density in three patients (9%), short stature in 2 patients (6%) and cataract in 2 patients (6%) developed. Conclusions: Children with nephrotic syndrome carry a risk in terms of short stature, osteoporosis, cataract and renal failure in the long-term follow-up. It was observed that our rates of response to steroid were similar to the literature and the most common histopathological diagnosis was focal segmental glomerulosclerosis in our patients who underwent biopsy because of resistance to steroid. It was thought that multi-center studies should be conducted to demonstrate regional or national differences related with long-term results of childhood nephrotic syndrome. (Türk Ped Arş 2015; 50: 37-44)

Idiopathic Nephrotic Syndrome in Childhood: A Retrospective Analysis of Two Hundred and Eighty Nine Patients

“…In the current study, the rate of response to steroid was found to be 81.7%. Resistance to steroid was detected in 18.3% of children with INS which was similar with that in a previous study performed in our country (5).…”

Nefrotik Sendrom Tanılı Çocukların Son Beş Yılda Hastaneye Yatış Endikasyonları ve Prognozları

“…Sadece steroide dirençli NS tanılı çocuk hastaların alındığı bir çalışmada ise hastaların %26.8'inde evre 5 KBH geliştiği bildirilmiştir (16). Ülkemizden bildirilen bir çalışmada ise 3 yıllık takip sonunda kronik böbrek yetmezliği oranı %7.7 iken 5 yıllık takip sonunda bu oran %9 olarak bildirilmiştir (8). Çalışmalardaki evre 5 KBH gelişen hastaların oranlarındaki farklılıkların çalışmaya alınan hastaların farklı gruplardan (yatan hastalar, sadece steroide dirençli NS tanılı hastaların vb.)…”

Review on long‐term non‐renal complications of childhood nephrotic syndrome

“…As mentioned earlier, glucocorticoids inhibit osteoblastogenesis and stimulate osteoblast apoptosis, which can then lead to impaired bone formation . When steroids are administered, osteoclast numbers and bone resorption are rapidly increased .…”

“…As mentioned earlier, glucocorticoids inhibit osteoblastogenesis and stimulate osteoblast apoptosis, which can then lead to impaired bone formation. 25 When steroids are administered, osteoclast numbers and bone resorption are rapidly increased. 26 Together with osteoblast inactivation and decreased bone formation, these changes lead to the loss in bone mineral density (BMD).…”